{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,20]],"date-time":"2025-09-20T02:09:34Z","timestamp":1758334174091,"version":"3.44.0"},"reference-count":4,"publisher":"Oxford University Press (OUP)","issue":"9","license":[{"start":{"date-parts":[[2025,8,20]],"date-time":"2025-08-20T00:00:00Z","timestamp":1755648000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100005739","name":"Universidad Nacional Aut\u00f3noma de M\u00e9xico","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100005739","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,8,29]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Background<\/jats:title>\n                  <jats:p>Parkes Weber syndrome (PWS) is a rare congenital vascular syndrome characterized by complex capillary malformation , venous malformation, lymphatic malformation, and arteriovenous malformation (AVM) in the affected limb with overgrowth; the latter is a pathognomonic feature that differentiates it from Klippel\u2013Trenaunay syndrome. Cardiovascular complications include increased cardiac output, which promotes the onset of heart failure and the development of pulmonary hypertension (PAH), significantly impairing the quality of life due to severe functional class deterioration. However, these complications are currently treatable by ligation or removal of malformations.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Case report<\/jats:title>\n                  <jats:p>A 33-year-old male with a long-standing, progressively enlarging AVM of the right upper limb presented with necrosis and haemorrhage, leading to hypovolemic shock. Angiography revealed an AVM involving the subclavian, axillary, and brachial arteries, necessitating embolization and surgical interventions. Six years later, he developed distal finger necrosis, requiring infracondylar amputation. He later presented with stump infection, purulent discharge, orthopnoea, jugular venous distension, a loud second heart sound, and a holosystolic murmur in the tricuspid region. Echocardiography and catheterization confirmed PAH and a high-flow arteriovenous fistula. Multidisciplinary evaluation led to definitive amputation and PAH treatment. Postoperatively, PAH resolved, and the patient was discharged with ongoing follow-up, showing significant improvement.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Discussion<\/jats:title>\n                  <jats:p>This case report highlights the importance of a multidisciplinary approach in managing PWS, especially when endovascular interventions are not feasible owing to the diffuse nature of the AVM. It also emphasizes the potential for reversing severe complications through definitive surgical intervention in complex cases of PWS.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/ehjcr\/ytaf381","type":"journal-article","created":{"date-parts":[[2025,8,19]],"date-time":"2025-08-19T11:33:41Z","timestamp":1755603221000},"source":"Crossref","is-referenced-by-count":0,"title":["Parkes Weber syndrome, a rare case of pulmonary hypertension: a case report"],"prefix":"10.1093","volume":"9","author":[{"ORCID":"https:\/\/orcid.org\/0009-0000-4420-4644","authenticated-orcid":false,"given":"Camila","family":"Castillo-Tello","sequence":"first","affiliation":[{"name":"Department of Cardiology, National Institute of Medical Sciences and Nutrition Salvador Zubiran , Vasco de Quiroga 15, Belisario Dom\u00ednguez Secc 16, Tlalpan, Mexico City 14080 ,","place":["Mexico"]},{"name":"Faculty of Medicine, Universidad Nacional Aut\u00f3noma de M\u00e9xico , Circuito Interior, Avenida Universidad 3000, Ciudad Universitaria, Coyoac\u00e1n, Mexico City 04510 ,","place":["Mexico"]},{"name":"Heart Failure Clinic, Department of Cardiology, National Institute of Medical Sciences and Nutrition Salvador Zubiran , Vasco de Quiroga 15, Belisario Dom\u00ednguez Secc 16, Tlalpan, Mexico City 14080 ,","place":["Mexico"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Clemente","family":"Barron-Magdaleno","sequence":"additional","affiliation":[{"name":"Heart Failure Clinic, Department of Cardiology, National Institute of Medical Sciences and Nutrition Salvador Zubiran , Vasco de Quiroga 15, Belisario Dom\u00ednguez Secc 16, Tlalpan, Mexico City 14080 ,","place":["Mexico"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Consuelo","family":"Orihuela-Sandoval","sequence":"additional","affiliation":[{"name":"Heart Failure Clinic, Department of Cardiology, National Institute of Medical Sciences and Nutrition Salvador Zubiran , Vasco de Quiroga 15, Belisario Dom\u00ednguez Secc 16, Tlalpan, Mexico City 14080 ,","place":["Mexico"]},{"name":"Echocardiogram Subdivision, Department of Cardiology, National Institute of Medical Sciences and Nutrition Salvador Zubiran , Vasco de Quiroga 15, Belisario Dom\u00ednguez Secc 16, Tlalpan, Mexico City 14080 ,","place":["Mexico"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5833-0932","authenticated-orcid":false,"given":"Eduardo","family":"Guti\u00e9rrez-Le\u00f3n","sequence":"additional","affiliation":[{"name":"Division of Postgraduate Studies, Faculty of Medicine, Universidad Nacional Aut\u00f3noma de M\u00e9xico, Circuito de Posgrados s\/n, Ciudad Universitaria , Coyoac\u00e1n, Mexico City 04510 ,","place":["Mexico"]},{"name":"Department of Medical Education, National Institute of Medical Sciences and Nutrition Salvador Zubir\u00e1n, Vasco de Quiroga 15, Belisario Dom\u00ednguez Secc. 16 , Tlalpan, Mexico City 14080 ,","place":["Mexico"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2025,8,20]]},"reference":[{"key":"2025091917331530100_ytaf381-B1","doi-asserted-by":"crossref","first-page":"820","DOI":"10.1016\/j.avsg.2013.01.001","article-title":"Diagnosis and treatment of Parkes Weber syndrome: a review of 10 consecutive patients","volume":"27","author":"Gir\u00f3n-Vallejo","year":"2013","journal-title":"Ann Vasc Surg"},{"key":"2025091917331530100_ytaf381-B2","doi-asserted-by":"crossref","first-page":"959","DOI":"10.1002\/humu.20746","article-title":"Parkes Weber syndrome, vein of Galen aneurysmal malformation, and other fast-flow vascular anomalies are caused by RASA1 mutations","volume":"29","author":"Revencu","year":"2008","journal-title":"Hum Mutat"},{"key":"2025091917331530100_ytaf381-B3","doi-asserted-by":"crossref","first-page":"371","DOI":"10.1177\/0268355516664212","article-title":"Parkes Weber syndrome-diagnostic and management paradigms: a systematic review","volume":"32","author":"Banzic","year":"2017","journal-title":"Phlebology"},{"key":"2025091917331530100_ytaf381-B4","doi-asserted-by":"crossref","first-page":"168","DOI":"10.1177\/1534734620986683","article-title":"Major limb amputation in Parkes-Weber syndrome with refractory ulceration: a case report and literature review","volume":"22","author":"He","year":"2023","journal-title":"Int J Low Extrem Wounds"}],"container-title":["European Heart Journal - 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